Abstracts tagged "Skin fibrosis"

Abstract Number: 0551 • ACR Convergence 2021
Metabolic Intermediate Dimethyl-Alpha-Ketoglutarate Is a Novel Repressor of Pathogenic Myofibroblast Reprogramming and Skin Fibrosis in Systemic Sclerosis
Blaž Burja¹, Ishani Banik², Shervin Assassi³, Michael Whitfield⁴, J. Matthew Mahoney⁵, Andreja Erman⁶, Matija Tomšič⁷, Mitchell P. Levesque², Snežna Sodin-Šemrl⁷, Gabriela Kania⁸, Hubert Rehrauer⁹, Ziga Rotar⁷, Oliver Distler¹⁰, Katja Lakota¹¹ and Mojca Frank-Bertoncelj¹⁰, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich and Department of Rheumatology, University Medical Centre Ljubljana, Zurich, Switzerland, ²Department of Dermatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴Geisel School of Medicine, Lebanon, NH, ⁵Center for Quantitative Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ⁶Institute for Cell Biology, University Medical Centre Ljubljana, Ljubljana, Slovenia, ⁷Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, ⁸Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁹Functional Genomics Center Zurich, ETH Zurich and University of Zurich, Zurich, Switzerland, ¹⁰Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland, ¹¹Department of Rheumatology, University Medical Centre Ljubljana, Llubljana, Slovenia
Background/Purpose: Metabolic perturbations drive fibroblast-to-myofibroblast reprogramming and tissue fibrosis. Restoring perturbed metabolism might represent a new antifibrotic strategy. Here we explored the capacity of dimethyl-alpha-ketoglutarate…
Abstract Number: 1858 • ACR Convergence 2021
Serum Interferon Score Is a Biomarker of Active Disease in Patients with Early Diffuse Cutaneous Systemic Sclerosis Enrolled in the Prospective Registry of Early Systemic Sclerosis (PRESS) Cohort
Monique Hinchcliff¹, Suiyuan Huang², Shervin Assassi³, Elana Bernstein⁴, Flavia Castelino⁵, Robyn Domsic⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Ami Shah¹⁰, Victoria Shanmugam¹¹, Virginia Steen¹², Dinesh Khanna² and Francesco Del Galdo¹³, ¹Yale School of Medicine, Westport, CT, ²University of Michigan, Ann Arbor, MI, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴Columbia University, New York, NY, ⁵Massachusetts General Hospital, Boston, MA, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷University of Utah, Salt Lake City, UT, ⁸Hospital for Special Surgery, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Johns Hopkins Rheumatology, Baltimore, MD, ¹¹The George Washington University, Great Falls, VA, ¹²Division of Rheumatology, Georgetown University, Washington, DC, ¹³University of Leeds, Leeds, United Kingdom
Background/Purpose: Several published studies have demonstrated activation of the interferon type 1 (IFN) pathway in sera from patients with systemic sclerosis (SSc). Specifically, levels of…
Abstract Number: 025 • 2020 Pediatric Rheumatology Symposium
HLA Genetic Signatures Associated with Inflammatory Sub-type in Juvenile Localized Scleroderma
Christina Schutt¹, Emily Mirizio ², Kaila Schollaert-Fitch ² and Kathryn Torok ², ¹UPMC Children's Hospital of Pittsburgh, Pittsburgh, ²Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease of the skin and underlying tissue that is characterized by an earlier inflammatory infiltrate, followed by…
Abstract Number: 720 • 2019 ACR/ARP Annual Meeting
Baseline Subject Demographics and Disease Characteristics in a Phase 3 Study of Safety and Efficacy of Lenabasum in Diffuse Cutaneous Systemic Sclerosis
Robert Spiera¹, Nancy Dgetluck ², Bradley Bloom ², Barbara White ² and Christopher Denton ³, ¹Hospital for Special Surgery, New York, NY, ²Corbus Pharmaceuticals, Norwood, MA, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: We report the baseline characteristics of a large cohort of diffuse cutaneous systemic sclerosis (dcSSc) patients enrolled in a Phase 3 trial of lenabasum,…
Abstract Number: 865 • 2019 ACR/ARP Annual Meeting
Safety and Efficacy of Lenabasum at 21 Months in an Open-Label Extension of a Phase 2 Study in Diffuse Cutaneous Systemic Sclerosis Subjects
Robert Spiera¹, Laura Hummers ², Lorinda Chung ³, Tracy Frech ⁴, Robyn Domsic ⁵, Vivien Hsu ⁶, Daniel Furst ⁷, Jessica Gordon ¹, Madeline Myers ⁸, Robert W Simms ⁹, Elizabeth Lee ¹⁰, Scott Constantine ¹⁰, Nancy Dgetluck ¹⁰, Bradley Bloom ¹⁰ and Barbara White ¹⁰, ¹Hospital for Special Surgery, New York, NY, ²Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA, Baltimore, MD, ³Stanford, Palo Alto, Palo Alto, CA, ⁴Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ, ⁷University of California, Los Angeles, CA, ⁸University of Texas, Houston, Houston, TX, ⁹Boston University, Boston, MA, ¹⁰Corbus Pharmaceuticals, Norwood, MA
Background/Purpose: Lenabasum is a synthetic, non-immunosuppressive, selective cannabinoid receptor type 2 agonist that activates resolution of innate immune responses and limits fibrosis in animal models…
Abstract Number: 1042 • 2019 ACR/ARP Annual Meeting
Thy-1 (CD90) as a Novel Marker for Tracking in Vivo Skin Fibrosis
Benjamin Korman¹, Ananta Paine ², Stacey Duemmel ³, Marc Nuzzo ³ and Christopher Ritchlin ², ¹University of Rochester Medical Center, Rochester, NY, ²Division of Allergy, Immunology and Rheumatology, Center for Musculoskeletal Research, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA, Rochester, NY, ³University of Rochester, Rochester, NY
Background/Purpose: Thy-1 (CD90) is a cell surface marker which is found primarily on fibroblasts and whose expression has previously been shown to correlate with pathologic…
Abstract Number: 1043 • 2019 ACR/ARP Annual Meeting
The Metabolic Intermediate Alpha-Ketoglutarate Suppresses the TGFβ-driven Profibrotic Responses of Dermal Fibroblasts
Blaž Burja¹, Gabriela Kania ², Matija Tomšič ³, Tea Janko ³, Snezna Sodin-Semrl ³, Oliver Distler ⁴, Katja Lakota ³ and Mojca Frank-Bertoncelj ⁵, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, ²Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ³Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, LJUBLJANA, Slovenia, ⁴Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ⁵University Hospital Zürich, Zürich, Switzerland
Background/Purpose: Metabolic perturbations are emerging as drivers of fibroblast activation in fibrosis. Transcriptomic analyses have shown the enrichment of glycolysis and suppression of tricarboxylic acid…
Abstract Number: 1059 • 2019 ACR/ARP Annual Meeting
BDCA2 Targeting of Human Plasmacytoid Dendritic Cells via CBS004 Reverts Dependent IFN Activation and Tissue Fibrosis in vitro and in vivo
Rebecca Ross¹, Clarissa Corinaldesi ², Gemma Migneco ³, Yasser El-Sherbiny ⁴, Steve Holmes ⁵, Jörg Distler ⁶, Clive McKimmie ⁷ and Francesco Del Galdo ⁸, ¹Univesristy of Leeds, Leeds, England, United Kingdom, ²University of Leeds, Leeds, England, United Kingdom, ³University of Leeds, Leeds, United Arab Emirates, ⁴University of Nottingham, Nottingham, United Kingdom, ⁵Capella Bioscience, london, United Kingdom, ⁶Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ⁷University of Leeds, Leeds, United Kingdom, ⁸University of Leeds and LTHT NIHR Biomedical Research Centre, Leeds, United Kingdom
Background/Purpose: Human plasmacytoid dendritic cells (pDCs) have been implicated in the pathogenesis of Systemic Sclerosis (SSc) through their ability to infiltrate the skin and secrete…
Abstract Number: 1072 • 2019 ACR/ARP Annual Meeting
CCR2+ Circulating Monocytes Contribute to the Survival of ADSC in Bleomycin-Induced Skin Fibrosis
Madhavi Latha Chalasani¹, Liyoung Kim ¹ and Theresa Lu ², ¹Hospital for Special Surgery, New York, NY, ²Hospital for Special Surgery, Weill Cornell Medicine (Microbiology and Immunology), New York
Background/Purpose: Monocytes and monocyte-derived cells play a crucial role during homeostasis and also during the development of various inflammatory diseases including skin fibrosis. Ly6Chi inflammatory…
Abstract Number: 2693 • 2018 ACR/ARHP Annual Meeting
What Is the Effect of Cyclophosphamide Iv Pulse Therapy in Patients with Diffuse Cutaneous Systemic Sclerosis on Skin Involvement: An Observational Study
Brigit Kersten, Nathan den Broeder, Frank van den Hoogen, Els van den Ende and Madelon Vonk, Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands
Background/Purpose: Patients with systemic sclerosis who have proximal skin involvement are classified as diffuse cutaneous systemic sclerosis (DcSSc). Patients with progressive skin involvement have worse prognosis.…
Abstract Number: 1714 • 2017 ACR/ARHP Annual Meeting
Inhibition of Hedgehog Acyltransferase Alleviates the Profibrotic Effects of Transforming Growth Factor β in Systemic Sclerosis
Ruifang Liang¹, Rosebeth Kagwiria², Clara Dees³, Yun Zhang⁴, Oliver Distler⁵, Georg Schett⁶ and Jörg Distler⁷, ¹Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, ²Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, ³Department of Internal Medicine 3 and Institute for Clinical Immunology,, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, ⁴Department of Internal Medicine 3 and Institute for Clinical Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, ⁵Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁶Department of Internal Medicine 3 – Rheumatology and Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, ⁷Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany
Background/Purpose: Hedgehog acyltransferase (Hhat) catalyzes the attachment of the fatty acid palmitate onto Sonic Hedgehog (Shh), a modification essential for Shh signaling activity. Palmitoylation of…
Abstract Number: 2885 • 2017 ACR/ARHP Annual Meeting
Tadalafil Reduces Skin Fibrosis and Profibrotic Genes Expression in Patients with Systemic Sclerosis
Sakir Ahmed, Mohit Kumar Rai, Durga Prasanna Misra and Vikas Agarwal, Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
Background/Purpose: Currently, drugs that modify skin fibrosis in Systemic Sclerosis (SSc) have efficacy in certain subgroups of patients only. Phosphodiesterase-5 inhibitors (PDE5i) are known to…
Abstract Number: 2974 • 2015 ACR/ARHP Annual Meeting
Insulin Resistance Is Associated with the Digital Ulcer in Patients with Systemic Sclerosis
Seung-Geun Lee¹, Eun-Kyoung Park², Dong-Wan Koo², Geun-Tae Kim³, Hee-Sang Tag³ and Joung-Wook Lee⁴, ¹Division of Rheumatology, Department of Internal Medicine, Busan National University School of Medicine, Busan, South Korea, ²Division of Rheumatology, Department of Internal Medicine, Pusan National University School of Medicine, Busan, South Korea, ³Division of Rheumatology, Department of Internal Medicine, Kosin University College of Medicine, Busan, South Korea, ⁴Divsion of Rheumatology, Department of Internal Medicine, Busan St. Marys center, Busan, South Korea
Background/Purpose: Growing evidence supports the view that systemic sclerosis (SSc) is a vascular disease mediated by autoimmunity and evolving into progressive tissue fibrosis. Several vascular…
Abstract Number: 1697 • 2014 ACR/ARHP Annual Meeting
High Frequency Skin Ultrasound Detects Subclinical Diffuse Dermal Involvement in Patients with Limited Cutaneous Systemic Sclerosis
Alberto Sulli, Barbara Ruaro, Carmen Pizzorni, Giorgia Ferrari, Elena Bernero, Sabrina Paolino and Maurizio Cutolo, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy
Background/Purpose . The modified Rodnan’s skin score (mRSS) is the validated method to detect both severity and extension of skin involvement, and high frequency skin…

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